09 brain tumors in pediatrics v3

8/17/2019 09 Brain Tumors in Pediatrics v3

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Brain Tumors in Pediatrics

Resident Education

Lecture Series


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Brain Tumors - Background

20-30% of cancers in children

2500-3000 new diagnoses/year 2nd most common neoplasm

ost occur !efore age "0 years

ale/#emale $ "3/"0 &0-'0% 5 year sur(i(al


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Relati(e )ncidence of

*rain +umors in ,hildren

+a!le 25-"

ppro.imate

incidence of

common ,S

tumors in children

i11o oplac


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Location – Supra vs. Infra

Supratentorial 25-40% strocytoma low grade 6-20%

strocytoma high grade &-"2%

Ependymoma 2-5% i.ed glioma "-5%

7anglioglioma "-5%

8ligodendroglioma "-2%

E+ "-2%

,horoid ple.us tumor "-2%

eningioma "-2%

7erm ,ell +umors "-2%

8ther "-3%


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Location – Supra vs. Infra

)nfratentorial 45-&0%

edullo!lastoma 9E+: 20-25%

strocytoma low grade "2-"6%

Ependymoma 4-6%

*rain stem glioma high grade 3-;% *rain stem glioma low grade 3-&%

8ther 2-5%


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Brain Tumors - Signs/Symptoms

)ncreased intracranial pressure - symptoms


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Brain Tumors – Signs/Symptoms

)ncreased ), ? Signs

apilledema optic atrophy

Loss of (ision

8#, 9head circumference: increased

*ulging fontanelles spreading sutures

@Setting sunA sign 9arinaud syndrome:

)ncreased !lood pressure low pulse

herniationB


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Posterior Fossa & Brainstem

Tumors - !inica! Featuresosterior #ossa primary

ta.ia

+remors

=ysarthria

Stiff nec

apilledema

*rainstem primary

E.tremity weaness

,ranial ner(e signs ? dou!le (ision

? facial weaness

? swallowing

dysfunction


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"emisp#eric Tumors –

!inica! Features


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Treatment

Tumor Type Surgery XRT Chemo

edullo!lastoma CCC ,rSp CCC

Low grade astro CCC focal ---- cere!ellar CCC BBBB ----

optic glioma 8 BBBB BBBB


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Treatment - Surgery

)n general needed for diagnosis

- e.ceptionsD 7,+ *S7

)deal is gross total resection

*alance prognosis (s mor!idity =e!uling shunts reser(oirs

- for symptom/), reduction therapy


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Treatment – $adiation T#erapy

otential for use in all !rain tumors ? e.ceptionsD choroid ple.us tumors

euro-a.is prophyla.is 9cranio-spinal r.: ? if tumor disseminates (ia ,S#

,oncerns for long term effects ? neuro-cogniti(e

? hearing ? secondary cancers

? endocrine

? seletal growth


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T#erapy - #emot#erapy

dunct therapy in most cases ? particularly in 7,+ medullo!lastoma

8f interest in young children ? 9a(oid or prolong FR+:

*lood !rain !arrier may !e limiting

? ewer studies suggest this may not !e so ? Local deli(ery (ia pumps/reser(oir/)+


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%edu!!o!astoma/P'(T

Similar histology different tumor names !ased on location ? +herapies (ary

edullo!lastoma - posterior fossa

E+ - supratentorial

ineo!lastoma - pineal region

median age 5 years

D# $ 2D"

propensity to disseminate ? "/3 with metastatic disease at diagnosis


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%edu!!o!astoma

Prognostic Factors ge - Gounger tend to do worse

E.tent of resection

on-posterior fossa tumors on-locali1ed disease

Standard ris '0-60% 5 yr sur(i(al


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%edu!!o!astoma

,S# dissemination

? chec for leptomeningeal spread ? !rain/spine R) L

,an spread to lung li(er * !one LHs ? rare

=ifference !etween supratentorial E+ 9sE+:medullo!lastoma and pineo!lostomaB


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(pendymoma

"0% of childhood !rain tumorsedian age $ 3-4 yrs

2/3 of primary in posterior fossaay ha(e leptomeningeal spread - R)

of !rain/spine ,S#rognostic factorsD

? E.tent of resectionIII ? geD some reports of !etter sur(i(al if J 5-'years at diagnosis

?


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(pendymoma - continued

E.tent of resection most important

? ear to gross total resection 50-'5%

? Less than +R 0-30%

Radiation therapy helps sur(i(al

? Reduces local recurrence

,hemotherapy has not shown efficacy

Recurrence is rarely fi.a!le


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Brain Stem )!iomas

=iffuse intrinsic pontine gliomas ? median sur(i(al $ &-; months

? death within 2 years J ;0% ? Radiation - transient clinical impro(ement

Low grade gliomas

? tectal e.ophytic e.tra-medullary ? highly enhancing on R)

? more indolent


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Lo* )rade +strocytoma/)!ioma

30-35% of ,S tumors ? 40-50% supratentorial (irtually anywhere

D# $ 2D"

ssociation with #-" ? more indolent course

7+R J;0% 5 year sur(i(al

RF ? Radiation

? ,hemo if symptomatic progressi(e or recurrent


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Brain Tumors in , year o!ds

&0-'0% supratentorial

FR+ has significant neuro-cogniti(e effects

7oal of therapiesD ? =elay FR+ to at least 3 yrs old with chemotherapy

most relapse prior to FR+

,urrent study ?

Short course 9"& ws: chemo ? 2nd loo surgery

? #ocal 9conformal: FR+

? aintenance chemotherapy


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omp!ications From

Tumor/T#erapy eurological deficits

? lim! paresisK

Reha!/+/8+ support ? swallowing/speech dysfunctionK E+ Speech therapy

K utrition issues

? neuro-cogniti(e deficitsK School/education issuesK Social interaction issues

? endocrine dysfunction

? end-organ damage

K idney li(er hearing neuropathy


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From +BPertifying (am ontent ut!ine

Recognize the signs and symptoms of

craniopharyngioma

Recognize the clinical manifestations of brain tumor Recognize the physical characteristics of a

headache due to increased intracranial pressure

Differentiate the clinical manifestations of spinal cord

compression (eg, from a tumor) from those of othermyelopathies, and evaluate appropriately


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redits

Sachin ogal =

09 brain tumors in pediatrics v3

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